The Korean Journal of Hematology最新文献

{"title":"Molecular methods for genomic analyses of variant PML-RARA or other RARA-related chromosomal translocations in acute promyelocytic leukemia.","authors":"Min Jin Kim, John Jeongseok Yang, Claus Meyer, Rolf Marschalek, Tae Sung Park","doi":"10.5045/kjh.2012.47.4.307","DOIUrl":"https://doi.org/10.5045/kjh.2012.47.4.307","url":null,"abstract":"TO THE EDITOR: We read an interesting paper by Palta et al. in a recent issue of the Korean Journal of Hematology titled, \"ZBTB16-RARA variant of acute promyelocytic leukemia with tuberculosis: a case report and review of literature\" [1]. We would like to add some comments to their article and suggest additional molecular methods to confirm variant translocations in acute promyelocytic leukemia (APL). \u0000 \u0000Apart from its characteristic morphology, APL is strongly correlated with presence of the PML-RARA fusion gene due to a t(15;17) translocation. Patients displaying cryptic PML-RARA gene rearrangements or other translocations involving RARA are very rare. Since RARA generates chimeric fusion genes with various partners such as ZBTB16 at 11q23, NUMA1 at 11q13, NPM1 at 5q35, FIP1L1 at 4q12, and STAT5b at 17q11.2 (Fig. 1), confirmatory molecular methods to detect gene rearrangements are necessary when unique or unusual chromosomal translocations are found in APL cases. Palta et al. reported a case of APL accompanied by tuberculosis where cytogenetic study showed a variant chromosomal translocation, t(11;17)(q23;q21). First, although they describe this case as APL with t(11;17)(q23;q21) through bone marrow (BM), immunophenotyping, and cytogenetic studies, we noted an absence of results based on molecular methods. To demonstrate the existence of the ZBTB16-RARA fusion gene, a single specific reverse transcriptase-polymerase chain reaction (RT-PCR) with a specific primer to detect the gene rearrangement or multiplex RT-PCR (e.g. HemaVision) to detect ZBTB16-RARA could have provided an accurate molecular diagnostic result. Second, the authors' reference to a paper by Kang et al. was puzzling despite the fact that it was cited as a similar case with chromosomal translocations at a similar location, in cases of AML with chromosomal translocation t(11;17) [2]. In the first case, cytogenetic analysis from BM aspirate showed t(11;17)(q23;q21) in 18 out of 20 cells while the MLL probe in fluorescence in situ hybridization (FISH) showed split-out signals in 98.5% of interphase cells. The second case also showed t(11;17)(q23;q25) in the chromosome study while the FISH assay showed split-out signals in 79% of interphase cells. However, normal fluorescence patterns were found in 200 cells in additional FISH assays when a dual-color single-fusion PML-RARA probe was used for both cases, which led to the conclusion that the MLL gene was involved in the 11q23 breakpoint and RARA was not involved in the 17q breakpoint. Hence, the authors' citation of this study to explain the ZBTB16-RARA fusion gene appears inadequate, and a molecular study could unambiguously identify the involvement of ZBTB16 at 11q23 in their case. \u0000 \u0000 \u0000 \u0000Fig. 1 \u0000 \u0000Various RARA partner genes in acute promyelocytic leukemias. \u0000 \u0000 \u0000 \u0000We suggest the authors conduct RT-PCR analysis of the ZBTB16-RARA fusion gene if any remnant RNA or cDNA samples from their APL patient are available. However, if only ge","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"47 4","pages":"307-8"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2012.47.4.307","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31162318","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Measurements of treatment response in childhood acute leukemia.","authors":"Dario Campana,&nbsp;Elaine Coustan-Smith","doi":"10.5045/kjh.2012.47.4.245","DOIUrl":"https://doi.org/10.5045/kjh.2012.47.4.245","url":null,"abstract":"<p><p>Measuring response to chemotherapy is a backbone of the clinical management of patients with acute leukemia. This task has historically relied on the ability to identify leukemic cells among normal bone marrow cells by their morphology. However, more accurate ways to identify leukemic cells have been developed, which allow their detection even when they are present in small numbers that would be impossible to be recognized by microscopic inspection. The levels of such minimal residual disease (MRD) are now widely used as parameters for risk assignment in acute lymphoblastic leukemia (ALL) and increasingly so in acute myeloid leukemia (AML). However, different MRD monitoring methods may produce discrepant results. Moreover, results of morphologic examination may be in stark contradiction to MRD measurements, thus creating confusion and complicating treatment decisions. This review focusses on the relation between results of different approaches to measure response to treatment and define relapse in childhood acute leukemia.</p>","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"47 4","pages":"245-54"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2012.47.4.245","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31162866","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of previous invasive pulmonary aspergillosis on the outcome of allogeneic hematopoietic stem cell transplantation.","authors":"Ji Yean Lee,&nbsp;Chul Won Jung,&nbsp;Kihyun Kim,&nbsp;Jun Ho Jang","doi":"10.5045/kjh.2012.47.4.255","DOIUrl":"https://doi.org/10.5045/kjh.2012.47.4.255","url":null,"abstract":"<p><strong>Background: </strong>Invasive pulmonary aspergillosis (IPA) is one of the major complications encountered by patients receiving chemotherapy for hematologic malignancies. The prolonged period of intense immunosuppression following allogeneic hematopoietic stem cell transplantation (HSCT) may increase the risk of IPA recurrence in patients with a history of IPA. We evaluated the impact of a history of IPA on allogeneic HSCT outcome, and examined the incidence of IPA after HSCT.</p><p><strong>Methods: </strong>This retrospective study included 22 patients with a history of IPA prior to receiving allogeneic HSCT at the Samsung Medical Center from 1995 to 2007. Diagnosis of IPA was defined as proven (N=5), probable (N=0), or possible (N=17).</p><p><strong>Results: </strong>All 22 patients received amphotericin-based regimens to treat pre-transplant IPA. Secondary antifungal prophylaxis was administered to 10 patients during HSCT. The development of post-transplant IPA was observed in 2 patients. One of the patients died from septic shock within 2 days of the diagnosis of possible IPA. The other patient recovered from IPA, but eventually had a relapse of the primary disease. Of the 22 patients, the overall 2-year survival rate was 63% (95% confidence interval [CI]: 41-85), and the transplant-related mortality rate was 19% (95% CI: 0-38).</p><p><strong>Conclusion: </strong>Our results suggest that a history of IPA prior to HSCT does not have an adverse impact on transplant outcomes, although the small number of cases was a limitation in this study. Future studies involving a larger number of cases are needed to further examine this issue.</p>","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"47 4","pages":"255-9"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2012.47.4.255","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31162867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surgery in patients with congenital factor VII deficiency: A single center experience.","authors":"Shin-Hee Kim,&nbsp;Young Shil Park,&nbsp;Kee-Hwan Kwon,&nbsp;Jae Hoon Lee,&nbsp;Kwang Chul Kim,&nbsp;Myung Chul Yoo","doi":"10.5045/kjh.2012.47.4.281","DOIUrl":"https://doi.org/10.5045/kjh.2012.47.4.281","url":null,"abstract":"<p><strong>Background: </strong>Congenital factor VII (FVII) deficiency is a rare hemorrhagic disorder that can cause excessive bleeding during and after surgery in affected patients. The recombinant form of activated factor VII (rFVIIa, NovoSeven® from Novo Nordisk, Bagsvaerd, Denmark), which was developed as a second-generation bypassing agent, has recently been used in the management of bleeding for patients with congenital FVII deficiency.</p><p><strong>Methods: </strong>We reviewed the results of 8 surgical procedures in 5 patients with congenital FVII deficiency at the Kyung Hee University Hospital, Gangdong, Seoul, Korea, between January 2008 and June 2010. We administrated rFVIIa preoperatively in six patients and postoperatively in five patients.</p><p><strong>Results: </strong>Between January 2008 and June 2010 at our center, 8 operations were performed successfully and no complications were observed in the 5 patients with congenital FVII deficiency. The median level of FVII activity was 2% (range, 0.6-7%). Four orthopedic procedures, 1 tonsillectomy, and 3 dental extractions were performed. The median duration of hospitalization was 8.5 days (range, 0-15 days). rFVIIa was administered at all procedures, except the dental extraction that was performed using only antifibrinolytic agents without any replacement. No bleeding or thrombogenic complications were observed in any case.</p><p><strong>Conclusion: </strong>Patients with congenital FVII deficiency who require surgery can be treated efficiently and safely with rFVIIa or antifibrinolytic agents. rFVIIa was well tolerated and maintained effective hemostasis and showed good clinical outcome after the major surgery.</p>","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"47 4","pages":"281-5"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2012.47.4.281","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31162313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Phenotypic consensus markers for plasma cell myeloma.","authors":"Mina Hur","doi":"10.5045/kjh.2012.47.4.239","DOIUrl":"https://doi.org/10.5045/kjh.2012.47.4.239","url":null,"abstract":"The clinical relevance of flow cytometric immunophenotyping (FCM) has been established in the diagnosis, classification, and monitoring of disease in monoclonal gammopathies [1, 2]. Clinical application of FCM encompasses differential diagnosis of malignant plasma cell disorder from reactive plasmacytosis, identifying the progression risk in monoclonal gammopathy of undetermined significance and in asymptomatic plasma cell myeloma, and minimal residual disease detection [1, 3]. Although most of the plasma cells in patients with multiple myeloma are neoplastic myeloma cells, a small percentage of normal or reactive plasma cells remain, which are responsible for maintaining normal immune function [4]. Reactive plasma cells are characterized by low forward/side scatter (FSC/SCC) and high CD38 expression together with a CD19+/CD56- phenotype. On the contrary, neoplastic myeloma cells are CD19-/CD56+ or CD56-, with high FSC/SCC and relatively low CD38 expression [5-7]. \u0000 \u0000The most commonly used antigens for the detection of neoplastic and normal plasma cells include CD19, CD56, CD20, CD117, CD28, CD33, CD27, CD81, CD31, CD39, CD40, CD44, cyclin D1, and CD34. It is impossible to define plasma cells as being phenotypically abnormal using only one test antigen either at diagnosis or after treatment, and there has been no study to identify the minimum requirements for reproducible detection of minimal residual disease [3]. According to the European Myeloma Network report, CD38, CD138, and CD45 should all be included in at least one tube for plasma cell identification and enumeration, and the primary gate should be based on CD38 vs. CD138 expression. The combined use of CD19 and CD56 was suggested as a minimal panel for the detection of abnormal plasma cells, which can be applicable to at least 90% of patients with multiple myeloma. A preferred panel that includes CD20, CD117, CD28, and CD27 was also suggested, which can be applicable to more than 95% of such patients [3]. \u0000 \u0000In the current issue of the Korean Journal of Hematology, Jeong et al. report a simplified FCM panel for multiple myeloma [8]. The authors suggest that a simplified immunophenotypic panel, CD56/CD19/CD138 (CD38)/CD45, is useful for distinguishing neoplastic myeloma cells from reactive plasma cells at diagnosis and during follow-up of patients with multiple myeloma. They also demonstrate that the negative expression of CD19 is the most valuable tool for identifying neoplastic myeloma cells in these patients. \u0000 \u0000The construction of an immunophenotypic panel for the diagnosis and follow-up of multiple myeloma is a matter of choice in the clinical laboratory. From a practical point of view, it would be ideal if a simple but cost-effective panel were applicable to almost all cases. In this regard, the study by Jeong et al. provides a practical solution that can be used both for the primary gating and for the differentiation between neoplastic myeloma cells and reactive plasma cells. The us","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"47 4","pages":"239-40"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2012.47.4.239","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31162862","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A case of transfusion-related acute lung injury induced by anti-human leukocyte antigen antibodies in acute leukemia.","authors":"Sun Mi Jin,&nbsp;Moon Ju Jang,&nbsp;Ji Young Huh,&nbsp;Myoung Hee Park,&nbsp;Eun Young Song,&nbsp;Doyeun Oh","doi":"10.5045/kjh.2012.47.4.302","DOIUrl":"https://doi.org/10.5045/kjh.2012.47.4.302","url":null,"abstract":"<p><p>Transfusion-related acute lung injury (TRALI) is a noncardiogenic pulmonary edema that occurs during or within 6 hours after transfusion. Risk factors for TRALI, which is relatively common in critically ill patients, include recent surgery, hematologic malignancy, and sepsis. Here, we report a case of TRALI induced by anti-human leukocyte antigen (anti-HLA) class II antibodies (HLA-DR) occurring after transfusion of platelet concentrates in a patient with acute leukemia. Although most patients with TRALI show improvement within 48-96 hours, our patient's condition rapidly worsened, and he did not respond to supportive treatment. TRALI is a relatively common and serious adverse transfusion reaction that requires prompt diagnosis and management.</p>","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"47 4","pages":"302-6"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2012.47.4.302","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31162317","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Serum hepcidin levels and iron parameters in children with iron deficiency.","authors":"Hyoung Soo Choi,&nbsp;Sang Hoon Song,&nbsp;Jae Hee Lee,&nbsp;Hee-Jin Kim,&nbsp;Hye Ran Yang","doi":"10.5045/kjh.2012.47.4.286","DOIUrl":"https://doi.org/10.5045/kjh.2012.47.4.286","url":null,"abstract":"<p><strong>Background: </strong>Iron deficiency (ID) and iron deficiency anemia (IDA) are common nutritional disorders in children. Hepcidin, a peptide hormone produced in the liver, is a central regulator of systemic iron metabolism. We evaluated whether serum hepcidin levels can diagnose ID in children.</p><p><strong>Methods: </strong>Sera from 59 children (23 males and 36 females; 5 months to 17 years) were analyzed for hepcidin-25 by ELISA. Patients were classified according to hemoglobin level and iron parameters as: IDA, (N=17), ID (N=18), and control (N=24).</p><p><strong>Results: </strong>Serum hepcidin, ferritin, soluble transferrin receptor (sTfR), transferrin saturation, and hemoglobin levels differed significantly between groups (P<0.0001). Serum hepcidin and ferritin levels (mean±SD) were 2.01±2.30 and 7.00±7.86, 7.72±8.03 and 29.35±24.01, 16.71±14.74 and 46.40±43.57 ng/mL in the IDA, ID, and control groups, respectively. The area under the receiver operating characteristic curve for serum hepcidin as a predictor of ID was 0.852 (95% CI, 0.755-0.950). Hepcidin ≤6.895 ng/mL had a sensitivity of 79.2% and specificity of 82.8% for the diagnosis of ID. Serum hepcidin levels were significantly correlated with ferritin, transferrin saturation, and hemoglobin levels and significantly negatively correlated with sTfR level and total iron binding capacity (P<0.0001).</p><p><strong>Conclusion: </strong>Serum hepcidin levels are significantly associated with iron status and can be a useful indicator of ID. Further studies are necessary to validate these findings and determine a reliable cutoff value in children.</p>","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"47 4","pages":"286-92"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2012.47.4.286","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31162314","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Successful treatment of diffuse large B-cell lymphoma with clarithromycin and prednisolone.","authors":"Masashi Ohe,&nbsp;Satoshi Hashino,&nbsp;Atsuo Hattori","doi":"10.5045/kjh.2012.47.4.293","DOIUrl":"https://doi.org/10.5045/kjh.2012.47.4.293","url":null,"abstract":"<p><p>We report a case of diffuse large B-cell lymphoma (DLBCL) treated successfully with clarithromycin (CAM) and prednisolone (PSL). A 71-year-old woman presented with fever and cervical pain. DLBCL was diagnosed based on histological results from lymph node biopsy. Cervical pain was thought to be caused by the invasion of lymphoma cells into the cervical vertebrae. She initially received radiotherapy for the cervical lesion. She did not receive conventional chemotherapy because of the risk of recurrent non-tuberculous mycobacteria infection; therefore, she was treated with 20 mg/day PSL and 800 mg/day CAM to induce apoptosis in lymphoma cells. Complete remission was achieved after 6 months. The present findings suggest that CAM and PSL may be effective in some cases of DLBCL.</p>","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"47 4","pages":"293-7"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2012.47.4.293","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31162315","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Extramedullary blast crisis of secondary CML accompanying marrow fibrosis.","authors":"Jai Hyang Go,&nbsp;Joowon Park","doi":"10.5045/kjh.2012.47.4.243","DOIUrl":"https://doi.org/10.5045/kjh.2012.47.4.243","url":null,"abstract":"which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. A 54-year-old man was referred to our hospital with right flank pain. Three years ago, he was diagnosed with gastric mucosa-associated lymphoid tissue (MALT) lymphoma and successfully treated with radiotherapy. CBC showed a WBC count of 24. Bone marrow (BM) examination showed granulocytic and megakaryocytic proliferation with moderate dysplastic megakaryopoiesis (A; H&E stain, ×200), and diffuse reticulin fibrosis (B; reticulin stain, ×400). Primary myelofibrosis was the first diagnostic consideration after BM study. Chromosomal analysis, however, showed t(9;22)(q34;q11.2), indicating CML. Concurrent abdomen computerized tomography revealed enlarged inguinal lymph nodes. Inguinal lymph node biopsy showed diffuse infiltration of immature cells (C; H&E stain, ×400), which were positive for myeloperoxidase (D). BCR/ABL1 rearrangement was demonstrated by fluorescence in-situ hybridization analysis, and a diagnosis of granulocytic sarcoma (GS) was made. Accompanying extramedullary myeloid tumor, CML was classified as blastic phase. Secondary CML with a simultaneous manifestation of GS is rare. Combining morphological and molecular-cytogenetic approaches can help detect the coexistence of both neoplasms, especially in CML cases with fewer typical morphologic features.","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"47 4","pages":"243"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2012.47.4.243","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31162864","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary myelofibrosis and extramedullary blastic transformation with hemophagocytosis.","authors":"Misung Kim,&nbsp;Jooryung Huh","doi":"10.5045/kjh.2012.47.4.244","DOIUrl":"https://doi.org/10.5045/kjh.2012.47.4.244","url":null,"abstract":"which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. A 72-year-old woman presented with bleeding, swollen gums, and painful cervical lymphadenopathy. A CT scan revealed diffuse lymphadenopathy and hepatosplenomegaly. Initial laboratory tests showed the following: WBC level, 11.1 μg/mL; and a differential count with marked leukocytosis with a left shift. Bone marrow biopsy indicated prefibrotic myelofibrosis. There was no evidence of JAK2 or BCR/ABL mutation or Epstein-Barr virus load. Trisomy 8 mosaicism was detected (47, XY, +8[6]/46, XY[24]) on karyotyping. Excisional lymph node biopsy revealed immature myeloid cells admixed with mature myeloid components and occasional megakaryocytes (A: H&E, ×400). Most notably, there were numerous hemophagocytic macrophages (arrowheads). Blasts comprised 40% of the total cellularity and showed a mixture of strongly MPO-positive myeloblasts and MPO-negative, CD68-positive, and CD163-positive monoblastic cells. The patient was diagnosed with primary myelofibrosis and extramedullary blastic transformation (granulocytic sarcoma) with acute myelomonoblastic differentiation accompanied by hemophago-cytosis. Therefore, hydroxyurea chemotherapy was initiated. Hemophagocytosis can be seen in leukemic transformation of myelofibrosis.","PeriodicalId":23001,"journal":{"name":"The Korean Journal of Hematology","volume":"47 4","pages":"244"},"PeriodicalIF":0.0,"publicationDate":"2012-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5045/kjh.2012.47.4.244","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"31162865","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}